niv

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Thank you for viewing this presentation. We would like to remind you that this material is the property of the author. It is provided to you by the ERS for your personal use only, as submitted by the author. 2008 by the author

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Anita K Simonds Royal Brompton Hospital ERS NIV Course 2009

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Median survival 32 months from onset symptoms, 19 months from diagnosis (Aguila et al Neurol 2003;60:813-19) Poor prognostic factors: older age, any bulbar features at onset, lack of marital partner, short time from onset to diagnosis, gender 22% present with bulbar disease

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Anticipatory care plan Clinical assessment plan Symptom management Respiratory support Communication Nutritional support Depression, other determinants of qol Practical support/aids/family, carers Shared decision-making, Advance directives ie: Supportive care

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Symptoms, orthopnoea, SBD Spirometry Cough – cough peak flow Respiratory muscle strength Sleep study VC Sniff IP Lyall Neurol 2004

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Loss of REM sleep in patients with diaphragm dysfunction Arnulf et al Am J Respir Crit Care Med 2000: 161: 849-856 Preservation of phasic sternomastoid activity in some ALS patients

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Survival Qol SAQLI All patients Mod bulbar weakness Severe bulbar All patients Severe bulbar Mod bulbar No survival advantage in severe bulbar patients but qol improved

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A retrospective cohort study comparing survival from time of diagnosis in subjects who started NIV use when their FVC was ≥65% predicted (Early NIV) with subjects who started NIV when their FVC was below 65% predicted (Standard NIV). The Early group (n = 25) and the Standard group (n = 67) were similar except for pulmonary function (mean FVC in Early NIV group = 74.3±10.1% predicted and 48.3±11.3 in Standard group, p<0.001). The median time from ALS diagnosis to death was significantly longer in the Early NIV group (2.7 years vs. 1.8 years, p = 0.045). This remained significant after adjustment for potential confounding factors Survival from time of diagnosis was nearly 1 year longer in the Early group. ‘Our findings suggest that clinicians either encourage earlier use of NIV or use more sensitive tests for respiratory muscle impairment than upright FVC’.

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Forest plot from meta-analysis of three randomised, placebo controlled trials of riluzole in MND (ALS). The plot shows the effect on mortality at 12 months The result favours treatment with a risk of 0.78 Cf survival advantage with NIV=205 days

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Bourke et al AJCCRM 2001: Assessment pre, 1, 3, 5 month after starting NIV Generic: Improvements in GWbS (p=0.039), SF36 emotional limitation, health perception Specific: Improvements Epworth SS, SAQLI, CRDQ dyspnoea, fatigue & mastery Improvements at 1 month maintained at 5 months despite disease progression Indices of sleep-related symptoms most responsive Lyall et al Neurol 2001: NIV increased Vitality domain (SF36) by 25% for up to 15 months despite disease progression

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Extend survival Improve quality of life Treatment of an acute and reversible exacerbations (?prevention of exacerbations) Relief of dyspnoea Control of symptoms of sleep disordered breathings eg headaches, sleep fragmentation Allow effective treatment of other symptoms eg. opiates for pain without exacerbating respiratory failure, CO2 narcosis or oversedation Buying time to resolve affairs, say goodbye Improve concurrent resp/cardiac symptoms eg. COPD/ cardiac failure oedema Provide patient with sense of control/autonomy over endstage disease Ceiling of care in do-not-intubate (DNI) patients Intermediate step before tracheostomy ventilation

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Respiratory symptoms, SDB, orthopnoea, FVC < 50% (?65%), PCO2>6 Counselling, anticipatory plan on ventilatory support at diagnosis Trial of NIV/cough assist Ventilatory support declined Disease progression Daytime NIV plus cough assistance +/-PEG Disease progression Tracheostomy -IPPV Ceiling of non-invasive support Palliative care Withdrawal of ventilation Disease progression

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Bulbar dysfunction leading to aspiration Failure to control ventilation with NIV Failure to wean onto NIV following acute decompensation Combination of 24 hr ventilator dependence and reduced bulbar function Vocal cord dysfunction/upper airway problems/choking episodes Patient preference

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Addition of cough inexsufflator; Cough PF <160l/min, poor cough Teach pt and carer physio techniques, NIV assisted physio, ambu bag, portable suction machine

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Mustfa et al Neurology 2003;61:1285-7 Manual assistance PCF increased by 11% MI-E PCF increased by 26% in bulbar and 28% in non-bulbar pts (P=0.001) Greatest improvement seen in those with weakest cough

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Sancho et al Chest 2004: 125;1400-5 N=26, 15 with severe bulbar dysfunction MI-E able to generate effective PCF (>160l/min) for stable patients with ALS except for those with bulbar dysfunction who also have MIC > 1 l and PCF < 160l/min who probably have severe dynamic collapse of upper airway Stable patients with mild resp dysfunction and PCF > 240l/min might not benefit from M-IE during acute RTI Settings +40 to -40 cmH2O

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Impact of bulbar function - 2 types: those who can’t close glottis MIC=FVC in whom manually assisted cough cannot increase cough PEFR and those with unstable pharygeal walls MIC>1l Bulbar Non-bulbar

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Early dysphagia on clinical exam Speech & language (SALT) & nutritional assessment & advice. Dietary modification Symptom progression FVC > 80% Low risk FVC <80 >50% Mod risk FVC < 50% High risk PEG accepted: use experienced team: in all groups consider NIV use, Radiological insertion, or insertion under GA, avoid uncontrolled sedation at all costs PEG declined: Oral intake as tolerated, NG feeding, palliative hydration

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Traynor BJ et al J Neurol, Neurosurg Psych 2003 1258-1261 Median survival 7.5 months longer (bulbar 9.6 mths) for multidisciplinary ALS clinic group Attendance at ALS clinc independent covariate predictor of survival (p=0.02 Multidiscip Multidiscip

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Cramps: quinine sulphate 200mg bd, carbamazepine, phenytoin, magnesium, verapamil Laryngospasm, choking: avoid triggers, positional modification, sublingual lorazepam, cough assist, suction Emotional lability: explain, SSRIs, amitriptyline Spasticity: baclofen 10-80 mg daily, tizantidine 6-24 mg daily ?marijuana Pain: immobility, stiffness: positional support, NSAIDs, opiates, gabapentin, opiates (no concern if on NIV) Constipation: hydration, dietary advice, laxatives, amend analgesic regime Depression: psychological support, SSRIs, social network support, and support for carers Further reading: Leigh PN et al J Neurol Neurosurg Psych 2003;74 Suppl IV 32-47

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What is the most important issue in your life right now? What helps you to keep going? How do you see the future? What is your greatest worry or concern? Are there ever times when you feel down? If things got worse where would you like to be cared for? How would you like the an acute infective exacerbation managed? Home or hospital? Where would you prefer to be at the end of your life? Hospital, hospice or home?

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No direct evidence Advance Directives improve qol, however increase autonomy, and improve physician pt communication. Vast majority of patients with ALS approve of advance directives Physicians more likely to follow specific directives supported by discussion with patients than generalities Patients want to start discussion earlier than physicians, but tend to want physician to initiate discussion Preferences for life-sustaining measures may change over time (eg for ventilation), so discussions need to start early after diagnosis and continue

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A Fatal Complication of Noninvasive Ventilation To the Editor: The patient was a previously healthy 53-year-old man with amyotrophic lateral sclerosis who was started on nocturnal noninvasive positive-pressure ventilation (inspiratory pressure, 10 cm of water; expiratory pressure, 2 cm of water). He tolerated this well and decided that he did not want invasive mechanical ventilation in the future. The patient's disease progressed, but he continued to work full-time and used noninvasive positive-pressure ventilation all night and most of the day. He obtained a second ventilator, which he kept at work. More than a year after noninvasive ventilation was initiated, the patient's ventilating unit failed. The machine's error code indicated that there had been a power-supply failure. Respiratory distress quickly developed, and the patient was taken to a local hospital but died of respiratory failure before ventilation could be reinstituted. . Noah Lechtzin, M.D., M.H.S. Charles M. Weiner, M.D. Lora Clawson, M.S.N., C.R.N.P. Johns Hopkins University School of Medicine NEJM 344:533 2001 Number 7Baltimore, MD 21287

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Thorax 2006 Simonds AK

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82% pts felt had sufficient info 30% with mod to severe dysphagia had gastrostomy 17% breathing difficulties Only 28% with dyspnoea and 9.2% with FVC <40% pred receiving NIV 8% anxiety and 2.5% choking Advance Directives 90%, these followed in 97% Bradley WG et al Neurology 2001;57:500-504

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When to start, when to stop discussions Transportable ventilator, battery powered Autonomy: environmental aids, NIV masks easy to put on and off, ventilation compatible with wheelchair, anticipatory plan Suction machine Cough assist Home care strategy – outreach/community Risk management: ventilator breakdown, masks, filters tubing Daytime ventilation – mouthpiece, nasal interface Advanced planning

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Neudert C et al J Neurol 2001; 248:612-16

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Died peacefully G 88% UK 98% No patient (n=171) choked to death Breathless G 20% UK 30% Anxiety and restlessness G 8% UK 6% Choking on saliva or mucus G 7% UK 0% Coughing G 4% UK 20% Diffuse pain G 2% UK 2% Cf: peaceful geriatric deaths – 78.7% Neudert C et al J Neurol 2001; 248:612-16

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Acknowledging the end of the patient physician relationship Tendency to avoid as: hard to know exactly when death will occur, try to maintain hope, it’s difficult, worry about abandonment Patients may want to say goodbye - let them, give time and space, reciprocate Back AL et al Ann Int Med 2005:142;682-685

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NIV Nocturnal hypoventilation Daytime hypercapnia (don’t delay too long) Orthopnoea MIP <60% predicted Marked sleep fragmentation, daytime somnolence Early use ? VC<80% ?as physio adjunct Cough assist Weak cough not adequately helped by NIV Recurrent RTIs despite NIV Cough PEFR <160l/min Demonstratable improvement in cough using cough assist and good tolerance

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Thank you A.Simonds@rbht.nhs.uk